RT期刊文章SR电子T1 6分钟步行试验了软弱和步态异常过剩1缺陷综合症(P2.333)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP P2.333 VO 82是10补充A1米歇尔Blumenschine A1杰奎琳·蒙特斯A1 Ashwini R首页ao A1阿里扎改变A1 Kristin Engelstad A1 Darryl De Vivo年2014 UL //www.ez-admanager.com/content/82/10_Supplement/P2.333.abstract AB目的:评价6分钟步行试验(6 mwt)与步态分析作为一个结果测量过剩1缺陷综合症(Glut1DS)。背景:临床结果的措施需要Glut1DS评估潜在疗法的效果。6 mwt是一个被广泛接受的步行能力在不同神经疾病与出版规范标准。定量步态分析加上6 mwt可以识别疲劳和步态异常。设计/方法:21患者和21个控件完成了6 mwt步态分析;平均年龄分别为16.4和18.7年。Percent-predicted距离从规范计算值确定的弱点。疲劳被定义为距离衰减的第一个六分钟。步态参数包括步幅、速度、节奏、基础支持和percent-time上双的支持。配对样本t Glut1DS相比差异和控制。 Repeated measures ANOVA evaluated 6MWT and gait parameters over three time points in the two groups. RESULTS: Glut1DS patients (61.6%) walked shorter distances than controls (85.8%) (p<0.05). There was no measurable fatigue in either group. Glut1DS had lower velocities (p<0.0001), shorter stride lengths (p<0.0001), and spent more time in double support (p<0.001) than controls. Base of support for Glut1DS and controls was similar (p=0.11). Gait variables did not change significantly in controls, but variability in base of support increased during the six minutes in the Glut1DS group (p=0.009). CONCLUSIONS: Our studies show that Glut1DS patients are weaker than healthy controls. Fatigue is negligible. Glut1DS patients have impaired balance, spend more time in double support, and show increasing variability in base of support during the 6MWT. These disturbances reflect poor balance and weakness, and result in a decomposition of gait. Our findings indicate that the 6MWT with gait analysis captures key motor abnormalities in Glut1DS and may serve as an informative outcome measure in future clinical trials.Disclosure: Dr. Blumenschine has nothing to disclose. Dr. Montes has received personal compensation for activities with Isis Pharmeceuticals as a consultant. Dr. Rao has nothing to disclose. Dr. Alter has nothing to disclose. Dr. Engelstad has nothing to disclose. Dr. De Vivo has received personal compensation for activities with Isis Pharmaceuticals and Ultragenyx Pharmaceuticals.Tuesday, April 29 2014, 7:30 am-11:00 am